This episode combines two articles and discusses the importance of laboratory expertise in accurately diagnosing autoimmune neurological syndromes, particularly autoimmune encephalitis and paraneoplastic syndromes, which are often difficult to identify due to overlapping symptoms and low prevalence. The author reviews two companion studies evaluating commercially available tissue-based assays for detecting antibodies against intracellular and surface antigens. The studies reveal significant limitations in the sensitivity and specificity of these commercial tests when used as standalone screening tools, highlighting the risk of both false positives and false negatives. Ultimately, the piece emphasizes the need for multimodal testing, skilled interpretation of results, and interdisciplinary communication between clinicians and laboratory staff to ensure reliable diagnosis and improve patient outcomes.

This paper provides a comprehensive review of repeat expansion disorders, focusing on their significance in neurology. It explains how abnormal expansions of DNA repeats cause a wide range of neurological conditions, highlighting the diagnostic challenges and opportunities for management. The authors discuss the molecular characteristics of these disorders, including repeat size, location, and inheritance patterns, and detail the clinical features like anticipation and heterogeneity that can aid in diagnosis. Finally, the piece outlines the evolution and current state of genetic testing methods, emphasizing the increasing role of whole-genome sequencing and bioinformatics tools in detecting these complex genetic variations.

This paper is a comprehensive review of IgG4-related disease (IgG4-RD) as it affects the nervous system. It discusses the clinical presentation, diagnosis, and treatment of this rare inflammatory and fibrotic condition, which can impact various parts of the nervous system, including the eyes, meninges, pituitary gland, brain parenchyma, and peripheral nerves. The article highlights the importance of early recognition and a high index of clinical suspicion due to the diverse ways IgG4-RD can manifest and mimic other conditions. While elevated serum IgG4 levels can be helpful, the paper emphasizes that histological confirmation is the gold standard for diagnosis. Treatment typically involves glucocorticoids as a first-line therapy, with other immunosuppressants like rituximab also being utilized, and a treatment algorithm for nervous system involvement is provided. The text also addresses the challenges in diagnosis due to the heterogeneity of symptoms and the ongoing need for further research and refined diagnostic criteria.

This paper presents the final findings from the extended phase of a clinical trial called CHAMPION MG, investigating the long-term effectiveness and safety of ravulizumab for adults with a specific type of myasthenia gravis characterized by antibodies against the acetylcholine receptor. The study followed patients for up to four years, including those who initially received ravulizumab and those who switched from placebo to ravulizumab. Results indicated sustained improvements in various measures of disease activity and quality of life for patients treated with ravulizumab. The research also highlights a significant reduction in corticosteroid use and clinical deterioration events among patients receiving the treatment, with no new safety concerns arising over the extended study period, including no reported cases of meningococcal infection.

This text summarizes interim results from the Second European Carotid Surgery Trial (ECST-2), a multicenter randomized trial comparing optimized medical therapy (OMT) alone versus OMT plus revascularization for patients with asymptomatic or low-to-intermediate risk symptomatic carotid stenosis of 50% or greater. The trial used the Carotid Artery Risk (CAR) score to identify patients predicted to have a 5-year stroke risk of less than 20%. The 2-year interim analysis, utilizing a hierarchical outcome and win ratio method, found no significant evidence that adding revascularization to OMT provided a benefit in this patient population. The study suggests that OMT alone might be sufficient for this group, but notes that longer-term 5-year data is needed for confirmation.

Published in New England Journal of Medicine the study details the MINT trial, a phase 3 clinical trial evaluating the efficacy and safety of inebilizumab for treating generalized myasthenia gravis (MG). #Neurology #Myasthenia

Brain MRI pattern as prognostic factor in Anti NMDA Encephalitis #Neurology, #MS, #MRI

Efgartigimod Efficacy and Safety in CIDP - ADHERE and ADHERE+ Trials #Neurology #CIDP #Efgartigimod